Girl with tough spirit, fragile skin sheds bandage cocoon

With skin so tender that it tears with touch. How a Phoenix family copes with their 3-year-old's rare disease.
KPNX-TV, Phoenix

Amy B Wang, The Arizona Republic
8:34 p.m. EST December 26, 2013

Lizzy Hendrickson, 4, was born with 'butterfly disease': Her skin tears at the slightest touch.

Lizzy Hendrickson, left, leans over to kiss her mom, Kristin, on Dec. 20, 2013, in their Phoenix home. She suffers from epidermolysis bullosa, also known as the butterfly disease, because her skin is extremely fragile. She recently stopped using bandages on her legs to protect her skin.(Photo: Michael Chow, The Arizona Republic)

Story Highlights

Rare disease called epidermolysis bullosa makes her skin so fragile that it blisters easily

EB affects 1 in 50,000 births, about as common as conjoined twins

It has no cure, no treatment, except to medicate and bandage the wounds

Lizzy was born with epidermolysis bullosa, or EB, a rare skin disease that causes skin to be so fragile it can blister at the slightest touch. People often refer to it as the "butterfly disease" because of how easily the delicate skin tears.

Even patients with the mildest forms of the disease need to bandage large areas of skin at all times. They constantly fear tearing the skin, which could lead to infection.

When the family's story first appeared in The Arizona Republic earlier this year, changing the dressings on Lizzy's wounds was a twice-daily task that involved multiple types of silicone foam bandages, gobs of Aquaphor ointment and rolls of elastic netting — and pain.

Still, Lizzy had already come a long way, breaking many EB "rules."

She usually bandaged only her legs. She attended a nearby preschool, where teachers knew how to dab rather than wipe when changing her diaper. Her parents wanted her to have as normal a life as possible.

The disease is so rare and has so many variations that it has no road map for treatment, according to Ronald Hansen, chief of dermatology at Phoenix Children's Hospital. Lizzy was transferred there a few hours after birth.

“Dr. (Ronald) Hansen has always said to us, 'You guys know best. If it makes sense to you, try it.'”

"We were all nervous," Rob Hendrickson said. Anytime Lizzy wanted to get on or off the couch, they picked her up. Jessie, the family dog, stayed outside.

"It was, 'Nobody touch Lizzy. Nobody bump into Lizzy,' " he said. The day ended without incident. "And we kind of looked at each other and said, 'That went pretty well.' "

They tried it again the next day, and the next, and the next — for a week and a half until summer vacation ended.

"Every day got a little more comfortable," Rob Hendrickson said. "I knew we were going to be OK by the last day before school started."

When Lizzy returned to preschool, her parents put her back in bandages. But a couple of weeks later, they tried the same thing, one day at a time.

By Labor Day, Lizzy had done the near unthinkable for an EB patient: She was bandage-free.

Instead of elaborate dressings, Lizzy now wears only Mepilex Border dressings on open wounds or blisters. The patches cover but do not adhere to the skin like regular bandages.

Lizzy Hendrickson's skin is extremely fragile because she suffers from epidermolysis bullosa, also known as butterfly disease.(Photo: Michael Chow, The Arizona Republic)

The change has had an unexpectedly positive ripple effect: Lizzy has no more painful dressing changes, no more elastic netting that might stick to her skin. Bath time used to be a drawn-out affair that produced tears and howls heard throughout the house.

"Now, it's 'OK, let's take a bath,' " Kristin Hendrickson said. "Sometimes we have a mini-battle over that, but it's not like it was before."

A few weeks after going bandage-free, Lizzy also stopped taking Doxepin, a drug intended to stop itching. Her mom has noticed a transformation.

"She was just kind of zonked out every morning," Kristin Hendrickson said. "She seems happier to me now."

Lizzy has had some bumps in the road. She started taking gymnastics, just like sister Katie, 7. During an early class, she came home with searing blisters on her palms, the result of trying to hang from a bar.

"That was not good," Rob Hendrickson said.

However, a week later Lizzy returned to the bars — with coaches supporting most of her weight. Now, three months later, her dad often finds her hanging from monkey bars on her own, and it doesn't bother her palms at all.

It's as if her skin is changing, becoming more resilient.

Rob and Kristin Hendrickson don't question why. They believe that Lizzy needed the dressing changes during the early years but also acknowledge going bandage-free is not a milestone for every EB patient because the disease is so mysterious.

“She's going to start getting more questions ... about what she has. If people ask her what's wrong, she has no idea what to say.”

The Hendricksons are still balancing confidence with caution, still hoping for a cure. They are considering participating in a clinical trial for a cream that could make wounds heal more quickly and less apt to blister. Meanwhile, they wonder how they can prepare Lizzy for questions people inevitably will ask.

"She's going to start getting more questions ... about what she has," Rob Hendrickson said. "If people ask her what's wrong, she has no idea what to say."

Lizzy turns 5 in May and likely will start kindergarten at a new school. Kristin Hendrickson worries whether other children will shun her and whether the marks and scars from EB will have any long-lasting psychological effect.

"That's certainly the biggest concern right now," Rob Hendrickson said. "Physically, she's self-regulating. She knows what she can do and what she can't do. ... If she is a confident kid, that'll help with anything else."

On a recent day, Lizzy wore only three patches: one on her right foot and two on her left leg, none of which bothered her as she twirled around her living room.

At 4½, Lizzy's vocabulary has grown exponentially, and she enjoys pointing out her newly painted pink room with a real twin bed handed down from her older sister.

She's learning to do simple addition and recently mastered 5 + 4 using her fingers. Her favorite color is now "pink and purple and blue and orange."

"I can show you a magic trick!" she says, taking a reindeer eraser and rubbing it between her palms to make it "shrink."

Older sister Katie asks if Lizzy wants to put on a dance show — the girls' latest favorite activity — and they screech and giggle as they dance to What Does the Fox Say? on repeat, chasing each other in circles.

Her dad stops Lizzy as she's performing a series of careful cartwheels.